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肺泡蛋白沉积症:两名患者的长期自发缓解

Pulmonary alveolar proteinosis: prolonged spontaneous remission in two patients.

作者信息

Martínez-López M A, Gómez-Cerezo G, Villasante C, Molina F, Diaz S, Cobo J, Medraño C

机构信息

Dept. of Internal Medicine, Hospital La Paz, Universidad Autónoma de Madrid, Spain.

出版信息

Eur Respir J. 1991 Mar;4(3):377-9.

PMID:1864355
Abstract

Pulmonary alveolar proteinosis is a rare idiopathic diffuse airspace disease characterized by intra-alveolar accumulation of large quantities of lipoproteinaceous material, with preservation of the lung interstitium. The clinical course of pulmonary alveolar proteinosis is variable. Spontaneous resolution is known to occur in up to a quarter of the cases. We describe two patients with untreated pulmonary alveolar proteinosis who experienced complete clinical, functional and radiographic resolution. In follow-up periods of fourteen and six years, both patients have remained asymptomatic.

摘要

肺泡蛋白沉积症是一种罕见的特发性弥漫性气腔疾病,其特征是肺泡内积聚大量脂蛋白物质,而肺间质保持完好。肺泡蛋白沉积症的临床病程多变。已知多达四分之一的病例会自发缓解。我们描述了两名未经治疗的肺泡蛋白沉积症患者,他们在临床、功能和影像学上均完全缓解。在14年和6年的随访期内,两名患者均无症状。

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